Alternative Names

Causes

Doctors do not know what causes idiopathic pulmonary fibrosis (IPF) or why some people get it. Idiopathic means the cause is not known. The condition may be due to the lungs responding to an unknown substance or injury. Genes may play a role in developing IPF. The disease occurs most often in people between 50 and 70 years old.

When you have IPF, your lungs become scarred and stiffened. This makes it hard for you to breathe. In some people, IPF gets worse quickly over months or a few years. In others, IPF worsens over a much longer time.

Symptoms

Shortness of breath during activity (this symptom lasts for months or years, and over time may also occur when at rest)

Exams and Tests

The health care provider will perform a physical exam and ask about your medical history. You will be asked whether you have been exposed to asbestos and if you have been a smoker.

The physical exam may find that you have:

Abnormal breath sounds called crackles

Blue-colored skin (cyanosis) around the mouth or fingernails due to low oxygen (with advanced disease)

Enlargement of the fingernail bases, called clubbing (with advanced disease)

Patients with idiopathic pulmonary fibrosis have abnormal breath sounds called crackles. Patients with advanced disease may have blue-colored skin (cyanosis) around the mouth or in the fingernails due to low oxygen.

Examination of the fingers and toes may show abnormal enlargement of the fingernail bases (clubbing).

Tests that help diagnose idiopathic pulmonary fibrosis include the following:

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